WeightNameValue
1000 Titel
  • ClC-7 is a slowly voltage-gated 2Cl−/1H+-exchanger and requires Ostm1 for transport activity
1000 Autor/in
  1. Leisle, Lilia |
  2. Ludwig, Carmen F |
  3. Wagner, Florian A |
  4. Jentsch, Thomas |
  5. Stauber, Tobias |
1000 Erscheinungsjahr 2011
1000 LeibnizOpen
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2011-04-28
1000 Erschienen in
1000 Quellenangabe
  • 30(11): 2140-2152
1000 FRL-Sammlung
1000 Verlagsversion
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3117652/ |
  • https://doi.org/10.1038/emboj.2011.137 |
1000 Ergänzendes Material
  • http://emboj.embopress.org/content/30/11/2140#sec-20 |
1000 Publikationsstatus
1000 Begutachtungsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • Mutations in the ClC‐7/Ostm1 ion transporter lead to osteopetrosis and lysosomal storage disease. Its lysosomal localization hitherto precluded detailed functional characterization. Using a mutated ClC‐7 that reaches the plasma membrane, we now show that both the aminoterminus and transmembrane span of the Ostm1 β‐subunit are required for ClC‐7 Cl−/H+‐exchange, whereas the Ostm1 transmembrane domain suffices for its ClC‐7‐dependent trafficking to lysosomes. ClC‐7/Ostm1 currents were strongly outwardly rectifying owing to slow gating of ion exchange, which itself displays an intrinsically almost linear voltage dependence. Reversal potentials of tail currents revealed a 2Cl−/1H+‐exchange stoichiometry. Several disease‐causing CLCN7 mutations accelerated gating. Such mutations cluster to the second cytosolic cystathionine‐β‐synthase domain and potential contact sites at the transmembrane segment. Our work suggests that gating underlies the rectification of all endosomal/lysosomal CLCs and extends the concept of voltage gating beyond channels to ion exchangers.
1000 Sacherschließung
lokal structure–function
lokal trafficking
lokal antiport
lokal chloride channel
lokal conductance
1000 Fächerklassifikation (DDC)
1000 Liste der Beteiligten
  1. https://frl.publisso.de/adhoc/creator/TGVpc2xlLCBMaWxpYQ==|https://frl.publisso.de/adhoc/creator/THVkd2lnLCBDYXJtZW4gRg==|https://frl.publisso.de/adhoc/creator/V2FnbmVyLCBGbG9yaWFuIEE=|http://orcid.org/0000-0002-3509-2553|http://orcid.org/0000-0003-0727-6109
1000 Label
1000 Förderer
  1. Deutsche Forschungsgemeinschaft (DFG) |
1000 Fördernummer
  1. JE 164/7
1000 Förderprogramm
  1. -
1000 Förderung
  1. 1000 joinedFunding-child
    1000 Förderer Deutsche Forschungsgemeinschaft (DFG) |
    1000 Förderprogramm -
    1000 Fördernummer JE 164/7
1000 Objektart article
1000 Beschrieben durch
1000 @id frl:6406566.rdf
1000 Erstellt am 2018-01-30T15:58:02.244+0100
1000 Erstellt von 218
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1000 Bearbeitet von 288
1000 Zuletzt bearbeitet Thu Aug 18 07:43:07 CEST 2022
1000 Objekt bearb. Fri Mar 05 09:13:02 CET 2021
1000 Vgl. frl:6406566
1000 Oai Id
  1. oai:frl.publisso.de:frl:6406566 |
1000 Sichtbarkeit Metadaten public
1000 Sichtbarkeit Daten public
1000 Gegenstand von

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